Bronchopulmonary Dysplasia (Chronic Lung Disease of Prematurity)

Bronchopulmonary Dysplasia (Chronic Lung Disease of Prematurity): Excerpt from The 5-Minute Pediatric Consult

John M. Good, MD

Bronchopulmonary Dysplasia - DIAGNOSIS

Bronchopulmonary Dysplasia - signs & symptoms

Bronchopulmonary Dysplasia - history

• Maternal use of antenatal steroids
• Gestational age, birth weight, APGAR score
• Initial resuscitative efforts, need for intubation, use of surfactant, duration in intubation, type of ventilation, duration of supplemental oxygen therapy, and other factors: These may have influenced the type and degree of lung injury.
• Familial history of asthma, atopy, or other children with bronchopulmonary dysplasia
• Social support structure
• Any potentially exacerbating factors, such as exposure to smoking

Bronchopulmonary Dysplasia - physical exam

• Review of systems, including careful assessment of work of breathing both at rest and during activity
• Feeding and sleeping history, and a review of growth charts
• Vitals including respiratory rate and pulse oximetry both at rest and with activity
• Signs of pulmonary hypertension, including peripheral edema, hepatomegaly, and venous distention

Bronchopulmonary Dysplasia - tests

• Changes on chest radiography include hyperinflation, emphysema, cyst formation, pulmonary edema, fibrosis, and cardiovascular changes. Severity of these changes may help predict the severity of the disease.
• Electrocardiogram: Often followed serially to assess for right ventricular hypertrophy
• Echocardiogram: Often a useful adjunct to follow patients with right ventricular hypertrophy
• Cardiac catheterization: Reserved for patients with evidence of pulmonary hypertension and cardiac dysfunction
• Pulmonary function testing: Often used to follow patients and evaluate responsiveness to interventions
• Blood gases: Useful in acute and chronic management of bronchopulmonary dysplasia to follow the degree of hypoxia and hypercapnia
• Bronchoscopy, barium swallow, pH probe, and sleep studies may reveal underlying conditions contributing to pulmonary dysfunction.

Bronchopulmonary Dysplasia - differencial diagnosis

• Asthma
• Bronchiolitis obliterans
• Congenital heart disease
• Cystic adenomatoid malformation
• Cystic fibrosis
• Idiopathic pulmonary fibrosis
• Infections
• Meconium aspiration syndrome
• Recurrent aspiration

Bronchopulmonary Dysplasia - TREATMENT

Bronchopulmonary Dysplasia - general measures

Bronchopulmonary Dysplasia - diet

• Infants with bronchopulmonary dysplasia may have increased caloric needs as much as 150 kcal/kg/d.
• Premature and critically ill infants may be deficient in antioxidants. Supplementation has not yet been shown to affect outcomes.

Bronchopulmonary Dysplasia - medication

• Diuretics:
  • Used for treating pulmonary edema, often improving lung mechanics and gas exchange
  • Furosemide may have other benefits, including effects on prostaglandin synthesis, direct vasodilatation, and improved surfactant production.
  • Side effects from long-term furosemide therapy include azotemia, ototoxicity, electrolyte abnormalities, excessive urinary calcium loss, osteopenia, and nephrocalcinosis.
  • Thiazide diuretics, usually used with a potassium-sparing diuretic such as spironolactone, are not as effective as furosemide.
  • Routine monitoring of electrolytes is recommended for patients on long-term diuretic therapy.
  • Electrolyte supplementation is often required with long-term diuretic usage.
• Bronchodilators:
  • Inhaled β-agonists are effective treatment for reversible bronchospasm, though safety and efficacy of long-term use has yet to be established.
  • Albuterol is often the drug of choice, though longer-acting agents are often used as well.
  • Muscarinic antagonists may be useful adjuncts, especially in patients who are not significantly responsive to albuterol. Believed to work on large and medium-sized airways
  • Cromolyn, though not a bronchodilator, is often used for its anti-inflammatory effects and has a low side-effect profile.
  • Methylxanthines are often used in the treatment of apnea, have a mild diuretic effect, and help improve diaphragmatic contractility, making them potentially useful in bronchopulmonary dysplasia.
• Pulmonary vasodilators:
  • Supplemental oxygen is an effective vasodilator and remains a mainstay of treatment for infants with hypoxia.
• Steroids:
  • Steroid usage is controversial.
  • Increased risk for sepsis has probably been overstated.
  • Often used successfully in short regimens to wean ventilatory support and hasten extubation
  • No long-term benefits of steroid therapy have been demonstrated.
  • Inhaled steroids may provide anti-inflammatory effects without systemic side effects, making them attractive as both prevention and treatment.
    • Routine use in premature infants is an active area of investigation.
    • Linear growth retardation has been a concern.
    • Newer agents that can be nebulized are now available, improving drug delivery in small infants.

Bronchopulmonary Dysplasia - FOLLOW UP

• A multidisciplinary approach is recommended for all patients with moderate and severe disease.
• Team may include primary care physician, pediatric pulmonologist, pediatric cardiologist, nutritionist, and speech, respiratory, occupational, and physical therapists.
• Monitor growth and nutritional status.
• Monitor neurodevelopmental status, including NICU “high-risk” follow-up.

• Ensure adequate calcium and phosphorus intake in patients at risk for hyperparathyroidism and rickets.
• Patients <2 years old are candidates for respiratory syncytial virus immune globulin injections (palivizumab; Synagis), if not contraindicated.
• Patients >6 months are candidates for influenza vaccine, if not contraindicated.
• Chest physiotherapy may cause pathologic fractures in patients with osteopenia.

Bronchopulmonary Dysplasia - prognosis

• Most survivors demonstrate slow, steady improvement.
• High death rate (17–47%) for patients with severe disease requiring prolonged mechanical ventilation
• No treatment modality has shown significant impact on the long-term outcome of chronic bronchopulmonary dysplasia.
• Survivors often have long-term pulmonary sequelae including hyperinflation, reactive airways, and exercise intolerance.
• Even older children and young adults who were thought to be asymptomatic can have abnormal responsiveness to exercise.
• Newer technologies, in particular high-frequency ventilation and exogenous surfactant, have improved survival rates for premature infants; however, reduction in the incidence and severity of bronchopulmonary dysplasia has been difficult to demonstrate.

Bronchopulmonary Dysplasia - complications

• Prolonged intubation may cause subglottic stenosis and tracheomalacia.
• Pulmonary hypertension may occur as a result of vasculature damage and subsequent intimal proliferation, which may, in turn, produce right ventricular hypertrophy and, if severe enough, cor pulmonale.
• Pulmonary edema often occurs secondary to increased pulmonary capillary permeability and increased pulmonary pressures.
• Reactive airways, bronchospasm, and altered pulmonary mechanics owing to a poorly compliant lung may result in abnormal pulmonary function testing and increased work of breathing.
• Malnutrition and growth failure may occur as a result of increased work of breathing and a subsequently high caloric expenditure.
• Impaired lung defenses result in an increased susceptibility to infection, especially respiratory syncytial virus.

Bronchopulmonary Dysplasia - bibliography

1. Bader D, Ramos AD, Lew CD, et al. Childhood sequelae of infant lung disease exercise and pulmonary function abnormalities after bronchopulmonary dysplasia. J Pediatr. 1987;10:693–699.
2. Bancalari E, Claure N, Sosenko IR. Bronchopulmonary dysplasia: Changes in pathogenesis, epidemiology and definition Semin Neonatol. 2003;8:63–71.
Hageman JR. The Pediatric Clinics of North America: Neonatology Update. Philadelphia, PA: WB Saunders; 1998.
3. Jobe AH, Ikegami M. Prevention of bronchopulmonary dysplasia. Curr Opin Pediatr. 2001;13:124–129.
4. Northway WH Jr, Rosan RC, Porter DY. Pulmonary disease following respiratory therapy of hyaline membrane disease. N Engl J Med. 1967;276:357–368.
5. Vaucher YE. Bronchopulmonary dysplasia: An enduring challenge. Pediatr Rev. 2002;23:349–358.

Bronchopulmonary Dysplasia - CODES

Bronchopulmonary Dysplasia - icd9

770.7 Bronchopulmonary dysplasia

Bronchopulmonary Dysplasia - FAQ

• Q: Will antibiotics help my child?
• A: Some evidence suggests that infection with ureaplasma may be important in the pathogenesis of bronchopulmonary dysplasia. It remains to be seen whether treatment affects outcome. Overuse of antibiotics increases occurrence of antibiotic resistance.
• Q: Which babies should get respiratory syncytial virus immune globulin injections (palivizumab; Synagis)?
• A: The AAP Committee on Infectious Diseases recommends immunoprophylaxis for infants with bronchopulmonary dysplasia who are <2 years old at the onset of respiratory syncytial virus season. Other premature infants may be candidates as well, with or without bronchopulmonary dysplasia:
• Infants born at ≤28 weeks at the onset of respiratory syncytial virus season and who are ≤12 months should receive immunoprophylaxis monthly for the entire season.
• Infants born at 29–32 weeks’ gestation and who are ≤6 months at the beginning of respiratory syncytial virus season should also receive immunoprophylaxis.
• Infants born between 32 and 35 weeks may or may not be candidates for palivizumab (Synagis) depending on the presence or absence of other risk factors, such as day-care attendance, school-aged siblings, exposure to environmental air pollutants, congenital abnormalities of the airways, or severe neuromuscular disease.
• Q: Will anti–respiratory syncytial virus immunoprophylaxis (palivizumab; Synagis) prevent my baby from getting respiratory syncytial virus?
• A: It won’t prevent respiratory syncytial virus, but it will help your child’s own immune system attack the virus.
• Q: Will my child have asthma when he grows up?
• A: Asthma occurs in >50% of older children who survived bronchopulmonary dysplasia.
• Q: What types of additional therapies can help my child?
• A: Such therapies include the following:
• Chest physiotherapy may help to mobilize secretions and to prevent atelectasis.
• Speech and occupational therapy may help infants who have had prolonged intubation or other interventions that interfere with oral functioning (and so may have some degree of oral-motor dysfunction and oral aversion).
• Other infants simply with increased work of breathing may have discoordinated suck and swallow, making oral feedings difficult.
• Physical therapy may help infants with gross and fine motor delays, poor tone, and abnormal posture.
• Parents can learn many of the therapies to incorporate therapeutic exercises and positioning into their daily routines.

Book Source Details

• Book Title: The 5-Minute Pediatric Consult
• Author(s): M. William Schwartz MD; et al.
• Year of Publication: 2008
• Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.

More About Bronchopulmonary dysplasia

• Back to disease: Bronchopulmonary dysplasia: Introduction
• Next Book Extract About Bronchopulmonary dysplasia: Premature Adrenarche (The 5-Minute Pediatric Consult)
• More Book Extracts: All Online Books for Bronchopulmonary dysplasia

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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: The 5-Minute Pediatric Consult Authors: M. William Schwartz MD; et al. Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7577-9

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